Endolymphatic sac tumor with intralabyrinthine hemorrhage: a case report / 临床耳鼻咽喉头颈外科杂志

A 27-year-old female patient suffering endolymphatic sac tumor with intralabyrinthine hemorrhage was reported. The patient had hearing loss in the left ear with continuous tinnitus, and MRI showed the soft tissue shadow of endolymphatic sac. Considering that the tumor involved semicircular canal and vestibule,endolymphatic cyst tumor resection was performed by labyrinth route. After surgery, there was no cerebrospinal fluid leakage and facial nerve function was normal. More importantly, enhanced MRI of temporal bone showed no tumor recurrence 1 year after surgery.

Endolymphatic Sac Tumour: A Case Report and Review of the Literature

Introduction Endolymphatic sac tumor (ELST) is a slow-growing, low-grade, locallyinfiltrative tumor arising from the endolymphatic sac/duct, which is located in the posterior part of the petrous temporal bone. It may be sporadic in origin, or may be associated with Von-Hippel Lindau (VHL) syndrome. Case description A 40-year-old female patient with an ELST without VHL syndrome who was treated successfully by microsurgical extirpation of the tumor. Discussion We discuss the radiological features and the histopathology of this rare tumor and review the relevant literature. Conclusion The case herein reported adds to the previously-reported cases of this rare tumor.

Postoperative anxiety and its relationship with life quality in patients with Ménière's diseases / 中南大学学报(医学版)

To investigate the postoperative anxiety and its relationship with life quality in patients with Ménière's diseases.
 Methods: A total of 68 patients with Ménière's disease, who received the treatment of endolymphatic sac decompression from 2010 to 2016, were enrolled. They finished two scales (the self-rating anxiety scale and the quality of life questionnaire for endolymphatic sac decompression of Ménière's disease) by snail mail. The patients were divided into different groups based on their preoperative course, postoperative follow-up time, clinic stage and quality of life after endolymphatic sac decompression surgery, and their anxiety was analyzed. In addition, 109 gender and age-matched patients with other diseases of otorhinolaryngology served as the control group, and their anxiety was also analyzed.
 Results: There was no statistical difference in the postoperative anxiety among the patients with preoperative course for less than 1 year, 1 to 5 years or more than 5 years (all P>0.05). There was no statistical difference in the patients with different duration of follow-up (P>0.05). There was no statistical difference in the patients with the different clinic stages (all P>0.05). There was no statistical difference in the patients with the improved life or non-improved life after surgery (P>0.05). The degree of the anxiety in the Ménière's group was more severe than that in the control group (P<0.01).
 Conclusion: It needs to keep in mind that a certain degree of postoperative anxiety still keeps in patients with Ménière's disease.

Long-term results of endolymphatic sac mastoid drainage for Ménière disease / 临床耳鼻咽喉头颈外科杂志

To investigate the long-term efficacy of endolymphatic sac mastoid drainage for Ménière disease.Data from 26 patients diagnosed with MD strictly meeting the criteria issued by " Guideline of diagnosis and treatment of Meniere disease(2017)" from 2006 to 2015 were analyzed in this study.Endolymphatic sac mastoid shunt surgery was performed for each patient.The therapeutic effect was evaluated against the " Guideline of diagnosis and treatment of Meniere disease(2017)".Vertigo control and auditory function were measured after at least two years follow up.In 26 cases,16 cases were male and 10 cases were femaleThe age ranged from 24 to 71 years old,with an average of 52.04 years.The disease duration ranged from 1 to 32 years.22 cases were diagnosed as unilateral Ménière disease,and bilateral involvement was identified in 4 cases,thus a total of 30 ears were included.According to the preoperative staging of hearing,there were 0 cases in stage one,5 cases in stage two,16 in stage three and 9 cases in stage four.15 cases(57.7%)achieved class A vertigo conrol(complete control),9 cases(34.6%)class B(substantial control)and 2 cases(7.7%)class D(no control).The severity of vertigo and its impact on daily life were improved in 24 cases(92.3%)with a score of 0 point,and 2 cases(7.7%)scored 2 points.Post-operative hearing was improved in 3 cases(11.5%),unchanged in 16 cases(61.6%)and worsened in 7 cases(26.9%).After operation,tinnitus disappeared in 5 cases(19.2%),reduced in 13 case(50%)and unchanged in 8 cases(30.8%).Endolymphatic sac mastoid drainage was an effective and safe management for intractable Ménière disease patients with pre-operative residual hearing.The occurrence of complication was unsual.The patients who are in stage four could gain benifits from the surgery.

Ménière's Disease and Electrophysiology / 대한평형의학회지

Inner ear is composed of cochlea, vestibule, and endolymphatic sac which are enclosed by thin layer of epithelial cells. The enclosed space is filled with fluid named as endolymph where the [K⁺] is high and [Na⁺] is low. This unique ion composition is very important in maintaining normal hearing and balance function by providing K⁺ ions into sensory hair cells, which finally depolarize hair cells to facilitate the transport of sound and acceleration stimulation to central nervous system. The ion composition of inner ear is maintained by various ion transport through ion channels, transporters, and exchangers in the inner ear sensory and extra-sensory epithelium. The disruption of normal endolymphatic ion composition by the deterioration of the function of those ion channels can cause dysfunction of sensory epithelium, which consequently results in hearing and balance disorders. One of the possible pathology from the disruption of inner ear ion homeostasis is endolymphatic hydrops which is a phenomenon of excessive fluid accumulation of inner ear. The dysfunction of ion channels in inner ear epithelium can be an etiology of Ménière's disease since endolymphatic hydrops is a main pathological finding of the disease. In this review, we discussed about the possible pathological mechanism of Ménière's disease as a perspective of channelopathy as well as the role of various ion channels in the regulation of inner ear fluid volume based on the findings revealed by electrophysiological studies.

Efficacy comparison between endolymphatic sac surgery and semicircular canal occlusion in the treatment of stage 4 Ménière disease / 临床耳鼻咽喉头颈外科杂志

OBJECTIVE@#To compare the vertigo controlling situation between the endolymphatic sac decompression(ELSD) and semicircular canal occlusion (SCO) in stage 4 Ménière disease.@*METHOD@#Fourteen patients who underwent endolymphatic sac decompression and 9 patients who underwent semicircular canal occlusion from 2009 to 2013 were followed. All patients has complete preoperative examination and postoperative follow-up.@*RESULT@#The vetigo control of the patients underwent endolymphatic sac decompression: completely control 35.7%; basic control 28.6%; partly control 14.3%; the vetigo control of the patients underwent semicircular canal occlusion: completely control 88.9%; basic control 11.1%.@*CONCLUSION@#ELSD and SCO are alternative methods for the patients of stage 4. SCO has a much highter complete vertigo control rate. For the patients without practical listening, SCO is a better choice.

A Case of Cholesterol Granuloma in the Nasal Septum / 대한이비인후과학회지

Cholesterol granuloma is rare and has occasionally been reported in uncommon sites of the head and neck, such as in the nasal sinus (frontal, ethmoid, sphenoid, or maxillary sinus), endolymphatic sac, and pterygoid process of the sphenoid sinus. The pathogenesis of thisnrare disease is not clear. We experienced one patient with cholesterol granuloma located in the nasal septum, which was managed successfully by endoscopic assisted excision.

Experiemental Model for Ménière's Disease Using Surgical Ablation of Endolymphatic Sac / 대한평형의학회지

Endolymphatic hydrops is a representing pathologic finding of Ménière's disease. For the induction of endolymphatic hydrops in an animal model, surgical ablation of endolymphatic sac has been used. Although traditional model with the blockage of endolymphatic sac induced severe hydrops, it has several limitations for the study of pathophysiology of Ménière's disease. Recently, modified experimental models have been introduced, in which additional procedure was performed to induce the acute aggravation of hydrops after the surgical ablation. These new models could be helpful to elucidate the mechanism and develop a new treatment of Ménière's disease. In this review, we introduce the characteristics of animal models using surgical ablation of endolymphatic sac from the classical model to novel modified models.

Changes in endolymphatic hydrops visualized by magnetic resonance imaging after sac surgery / 华中科技大学学报（医学）（英德文版）

The purpose of the study was to observe changes in endolymphatic hydrops by using intratympanic injection of gadolinium and magnetic resonance imaging (MRI) before and after endolymphatic sac surgery in patients with unilateral Meniere's disease. Thirteen patients with unilateral Meniere's disease undergoing endolymphatic sac surgery were retrospectively and prospectively analyzed. Three-dimensional fluid-attenuated inversion recovery or three-dimensional real inversion recovery MRI was performed 24 h after an intratympanic injection of gadolinium to grade the presence of endolymphatic hydrops. Among the 13 patients with hydrops confirmed by preoperative MRI, vestibular hydrops had no significant change in all patients; cochlear hydrops became negative in 2 patients, and remained unchanged in the other 11 patients after surgery. Definite vertigo attacks were substantially controlled in one patient and completely controlled in 12 patients during a follow-up period of 8-34 months after surgery. The hearing levels were improved in 3 patients, remained unchanged in 7 patients, and decreased in 3 patients. In conclusion, endolymphatic sac surgery does not always alleviate endolymphatic hydrops in patients with Meniere's disease. Relief from vertigo cannot always be attributed to the remission of hydrops. A change in hearing levels cannot be explained by hydrops status alone.

Efficacy of cochlear implantation for bilateral severe Ménière's disease / 临床耳鼻咽喉头颈外科杂志

OBJECTIVE@#To investigate the effecacy of cochlear implantation (CI) improving hearing of Lermoyez's syndrome, we retrospectively analyzed a case of Lermoyez's syndrome whose left ear was extremely severe sensorineural deafness and right ear was severe sensorineural deafness.@*METHOD@#The patient had completed preoperative audiological examination , vestibular function and imaging examination, then was carried out bilateral endolymphatic sac decompression and left side CI. Follow up after the surgery.@*RESULT@#The vertigo could be controlled very well after the bilateral endolymphatic sac decompression surgery, but the hearing loss couldn't be control, so CI was carried out to improve hearing and speech recognition rate. The contralateral hearing also improved significantly after the operation.@*CONCLUSION@#Endolymphatic sac decompression can effectively control. Ménière's disease in patients with vertigo attacks. CI can improve patients' hearing, and speech recognition rate and, also could improve the quality of life. CI was the first choice for the patient of 4 stage Ménière's disease.

A Case of Endolymphatic Sac Decompression of Intractable Meniere's Disease in Children / 대한이비인후과학회지

Meniere's disease in children is characterised with a hard and long-term diagnostic course, for which there is no accurate classification and treatment plan. A patient may undergo severe discomfort and long-term contraction of the disease. We experienced a 13-year-old female suffering from recurrent dizziness, and found that she had hearing loss, and tinnitus on her right ear. We diagnosed it as possible meniere's disease, but the disease progressed despite a trial of medication. The patient then underwent an endolymphatic sac decompression. During 6 months after the operation, she has shown only mild, intermittent dizzy symptoms, without severe vertigo. We report this clinical experience with a brief review of literature.

Tumarkin Otolithic Crisis Controlled by Endolymphatic Sac Surgery / 대한평형의학회지

Turmarkin otolithic crisis is a rare feature of Meniere's disease. It shows sudden falling to the ground with no warning sign. It is an otologic emergency because of the risk of falling, and it has traditionally been treated with labyrinthectomy or vestibular neurectomy. We experienced a 49-year-old male suffering from recurrent drop attack, and found that he had hearing loss, tinnitus or recurrent vertigo on his left ear, and could make a diagnosis him as Tumarkin otolithic crisis. We have performed the endolymphatic sac decompression, considering the hearing preservation and therapeutic opinion of patients. Two years after surgery, he showed intermittent, mild dizzy symptoms, without further drop attack. Therefore, we report our clinical experience with a brief review of literature.

Hemangioma of Endolymphatic Sac in Von Hippel-Lindau Patient / 대한이비인후과학회지

The present study reports a rare case of capillary hemangioma of endolymphatic sac. A 23-year-old male who underwent von Hippel-Lindau disease presented with recurrent sudden sensorineural hearing loss. Magnetic resonance imaging revealed a heterogenous enhanced mass in the right endolymphatic sac, which was hyperintense on the enhanced T1-weighted images and inhomogenous on the T2-weighted images. Pre-operatively, this tumor was believed to be an endolymphatic sac tumor because of the history of von Hippel-Lindau disease. During the surgery, vascular tumor was removed by transmastoid approach. A histopathological examination indicated that the tumor was a capillary hemangioma. To the best of our knowledge, the present study is the second case of hemangioma in the endolymphatic sac and first case of von Hippel-Lindau disease.

Efficacy of endolymphatic sac-mastoid shunt surgery for intractable Meniere's disease / 中华耳鼻咽喉头颈外科杂志

<p><b>OBJECTIVE</b>To investigate the effect and mechanism of endolymphatic sac-mastoid shunt surgery for intractable Meniere's disease of different stages according to hearing level.</p><p><b>METHODS</b>Data from 240 patients diagnosed with unilateral MD strictly meeting the criteria issued by Chinese Academy of Otolaryngology-Head and Neck Surgery Committee (2006) from January 1983 to January 2012 were analyzed in this study. Endolymphatic sac-mastoid shunt surgery was performed in affected ear for each patient. The evaluation of therapy followed the guidelines issued by Chinese Academy of Otolaryngology Head and Neck Surgery Committe. Vertigo control and auditory function were measured in two-year's follow-up.</p><p><b>RESULTS</b>According to the preoperative staging of hearing, among these 240 patients, there were 12 cases in stage I (with an average hearing threshold < 25 dB), 130 cases in stage II (with an average hearing threshold of 25-40 dB), 85 in stage III (41-70 dB) and 13 cases in stage IV(with an average hearing threshold > 70 dB). Overall control rate of vertigo was 77.9% (187/240) in two-year's follow-up, with total control 49.2% (118/240) and substantial control 28.7% (69/240). The hearing was improved in 25.0% (60/240) of cases, no change in 59.2% (142/240) of cases, and worse in 15.8% (38/240) of cases. According to different stages, vertigo control rate was 83.3% (10/12) in stage I, 82.3% (107/130) in stage II, 75.3% (64/85) in stage III and 46.2% (6/13) in stage IV. Vertigo control rate of stage IV patients was significantly lower than that of stage II and III patients (χ(2) = 9.318 and 4.692, P < 0.05), while vertigo control rate of stage I, II, III patients had no significant difference with each other (P > 0.05).</p><p><b>CONCLUSION</b>Endolymphatic sac-mastoid shunt operation is an effective method in the treatment of intractable Meniere's disease, but the effect was poor in stage IV patients.</p>

Current evidence for endolymphatic sac surgery in the treatment of Meniere's disease: a systematic review

This paper aims to identify emerging evidence for endolymphatic sac surgery (ESS) in the treatment of Meniere's disease since the landmark study by Thomsen et al, published in 1998 (conducted from 1981 to 1989). Using the MEDLINE database (PubMed), a systematic review of the literature published from January 1990 to June 2014 was performed. We included all English-language, peer-reviewed randomised controlled trials (RCTs) and controlled studies. Single-arm cohort studies were included if the sample size was ≥ 90 with a response rate > 60%. Altogether, 11 studies fulfilled our inclusion criteria; one was an RCT, two were controlled trials and eight were single-arm cohort studies. There currently exists a low level of evidence for the use of ESS in the treatment of Meniere's disease. Further studies, in particular RCTs and/or controlled studies, are required to fully evaluate this modality. However, there are difficulties in designing a valid placebo and achieving adequate blinding of observers and investigators.

Profound Hearing Loss after Endolymphatic Sac Decompression in Intractable Meniere's Disease / 대한평형의학회지

Endolymphatic sac decompression (ESD) is indicated in intractable Meniere's disease patients with serviceable hearing. A 43-year-old man presented with recurrent vertigo and fluctuating right hearing loss that had been intractable to medical treatment. ESD was performed for the purpose of vertigo control with hearing preservation. Positional vertigo with profound hearing loss developed immediate after surgery and positional vertigo was resolved within days. Following paralytic vestibulopathy with positive sign on head thrust test also resolved after 2 weeks, while sensorineural hearing loss was not recovered to preoperative level during 1 year of follow up. Recurrent vertigo attacks were developed again 1 year after the operation. Hearing preservation was not always guaranteed in ESD. Furthermore, chance of hearing loss should be included in informed consent though the procedure is purposed for hearing preservation.

A Case of Post-Traumatic Meniere's Disease

There are only a few reports of post-traumatic Meniere's disease and there is few literature that contains detailed data associated with the disease. We report a case of post-traumatic Meniere's disease. He suffered from tinnitus, fluctuating sensorineural hearing loss, and recurrent vertigo. Symptomatic medical treatment was not helpful and neither was soft tissue plugging around the oval and round windows during exploratory tympanotomy. Three months after soft tissue plugging, endolymphatic sac decompression surgery was performed. The patient's symptoms improved markedly thereafter. The clinical significance of post-traumatic Meniere's disease is described and we present a brief review of the literature.

Role of Endolymphatic Sac Decompression in Intractable Meniere's Disease / 대한평형의학회지

BACKGROUND AND OBJECTIVES: The role of endolymphatic sac decompression (ELSD) for the treatment of intractable Meniere disease (MD) has been debated. However, considering few treatment options for medically intractable MD with serviceable hearing or intractable bilateral MD, ELSD has shown reasonable treatment results without ablating inner ear function. The aims of this study are to review the role of ELSD in the treatment of MD and clinical course and long-term outcome after ELSD. MATERIALS AND METHODS: The ELSD was performed in 7 patients among 603 definite Meniere disease patients between May 2003 and December 2010. Patient's medical history and clinical courses after surgery were obtained by medical record review and telephone interview. RESULTS: Mean duration of follow up until receiving ELSD was 575 days since initial visit. Six patients showed complete control or substantial control of vertigo, but one patient suffered from sustained vertigo attacks even after ELSD, the vertigo was controlled after intratympanic gentamicin injection (ITGI) at 20 months after ELSD. Another patient had recurrence of vertigo after 30 months, which was successfully controlled by ITGI. The preoperatively mean monthly vertigo was 4.8 and it was significantly decreased to 1.5 after postoperatively 1 year, 0 after postoperatively 2 years (p<0.001). Hearing was preserved in 6 patients during the follow-up period. CONCLUSION: Most patients who were uncontrolled vertigo with 3 to 6 months medication showed significantly reduced vertigo and hearing preservation after ELSD. The ELSD seems to be a beneficial treatment option for intractable MD.

Endolymphatic sac tumor with von Hippel-Lindau disease: report of two cases with testing of von Hippel-Lindau gene / 中华耳鼻咽喉头颈外科杂志

<p><b>OBJECTIVE</b>Endolymphatic sac tumors (ELSTs) are rare in the general population with much higher prevalence in von Hippel-Lindau(VHL) disease. The purpose of this study is to present two cases of endolymphatic sac tumor with VHL disease with analysis of VHL gene and to explore their association with VHL disease using molecular analysis.</p><p><b>METHODS</b>Clinical data of these two patients from different VHL families were studied. DNAs extracted from peripheral bloods were amplified by the polymerase chain reaction using oligonucleotide primers corresponding to the VHL gene, then compared the mutations with the Human Gene Mutation Database.</p><p><b>RESULTS</b>In case 1, 6 family members were enrolled in the study. Among them, three had been identified to have a germline missense point mutation at codon 194 of the VHL gene exon 1 (p.S65W). The little sister of the patient (case 1) underwent vitrectomy for retinal hemangioblastoma 5 years ago in another hospital. The mother of the patient (case 1) was further diagnosed to have a cerebellar hemangioblastoma and renal carcinoma in the following physical examination. Case 2 with her parents were also tested. Codon 499 of the VHL gene exon 3 (p.R167W) were detected in case 2 and her mother, but the mother refused further examination.</p><p><b>CONCLUSIONS</b>The genetic diagnosis plays an important role in early detection of symptomatic patients and suspected patients. Clinical screening for members of the VHL families, and close follow-up of carriers allow an early detection of tumors and the metastasis, which is the most common cause of death of these patients.</p>